THE MAST CELL
Mast Cells are the oldest and one the most crucial immune cells, and are located in tissues, brain, joints, gastrointestinal tract, skin, cardio-vascular system and more. They play a key role in orchestrating our immune system with about 200 receptors and the storage, formation and selective (differential) release of more than 1,000 different mediators (e.g. inflammation, tissue development, fibrosis, atherosclerosis, autoimmunity, tumor growth etc.).
The multitude of activators and the many modes of mast cell response account for their ability to impact a variety of physiological and pathogenic processes. Receptor-binding agonists, physical activators and cell–cell contact can all activate mast cells. Activated mast cells can also respond by increasing the expression of ligands that mediate cell interactions with, for example, T and B cells. Responses to activation are heterogeneous and include the release of preformed mediators stored within granules and the synthesis and release of new mediators.
The dysfunction of the mast cells, for example chronic hyperactivity can lead to chronic inflammatory and autoimmune diseases.
Mast cell disease has long been thought to be just the rare disease of mastocytosis (in various forms, principally cutaneous and systemic), with aberrant mast cell mediator release at symptomatic levels due to neoplastic mast cell proliferation. The past decade of sciences show that a new view of mast cell disease is in order, with mastocytosis capping a “metaphorical iceberg” now termed Mast Cell Activation Disease (MCAD) and with the bulk of the iceberg being comprised of the more and more recognized Mast Cell Activation Syndrome (MCAS), featuring inappropriate mast cell activation leading to widespread systemic symptoms [2, 3, 4]. Depending on the individual occurrence and severity of symptoms patients with Mast Cell Activation Syndrome are often diagnosed with chronic illnesses such as irritable bowel syndrome (IBS), fibromyalgia, chronic fatigue syndrome / myalgic encephalomyelitis, various forms of allergy, chronic urticaria, chronic lyme disease and many more.
- Barbara Frossi, Francesca Mion et al., rheostatic functions of mast Cells in the control of innate and adaptive immune responses, trends in immunology, 2017 Apr 24, S1471-4906 (17) 30060-1
- Lawrence B. Afrin, Joseph H. Butterfield, Martin Raithel, and Gerhard J. Molderings, often seen, rarely recognized: mast cell activation disease – a guide to diagnosis and therapeutic options, annals of medicine, 2016
- Lawrence B Afrin, Gerhard J. Molderings, a concise, practical guide to diagnostic assessment for mast cell activation disease, World J Hematol 2014 February 6; 3(1): 1-17
- Gerhard J. Molderings, Stefan Brettner, Jürgen Homann, Lawrence B Afrin, mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options, Molderings et al. Journal of Hematology & Oncology 2011, 4:10